Prognosis

Favorable-histology Wilms tumors are associated with very high 5-year overall survival rates of >90%.[107][16][2]​​​​ However, there is an increased risk of treatment-related morbidity and mortality 25 years from diagnosis.[131]

Patients with tumor weight <550 g and age <2 years, in particular, have excellent outcomes and may be offered nephrectomy-only followed by imaging surveillance (according to the Children's Oncology Group [COG] treatment approach). These patients have a rate of cure near 90% with surgery alone and avoid the risk of adverse effects from adjuvant chemotherapy.[98][132][133]​​[134]

Presence of anaplasia, aneuploidy, distant metastatic disease (especially to the lymph node and/or hepatic metastasis and/or intravascular tumor thrombus), and loss of heterozygosity (LOH) at 1p and 16q, or gain of chromosome 1q in the tumor genetic profile confers poor prognosis.[32][33][58][59][79][107][117]​​​​​​​[123][135]​​​​​​​​[136][137][138]

Lymph-node involvement and the presence of microscopic residual disease are highly predictive of event-free survival and overall survival in patients with stage III favorable-histology disease.[139]

Prognosis is considered excellent in patients with stage IV disease if histology is favorable, metastatic disease is isolated to either the liver or lungs, and metastases responds to treatment with preoperative chemotherapy.[140] Worse outcomes are reported in patients with extrapulmonary metastases from favorable histology Wilms tumor compared to those with lung-only metastases.[106]​​ Prognosis is inferior for patients with high-risk stage IV tumors; the overall 5-year survival rates are 17% for blastemal type stage IV Wilms tumor and 16% for diffuse anaplastic stage IV Wilms tumor.[141]

Recurrence rates are low.[107] Adverse prognostic factors for patients with recurrent tumors include previous treatment with doxorubicin, relapses that occur <12 months after diagnosis and intra-abdominal relapse after abdominal irradiation.[107]​​[121][142]​​​ Prognosis for patients with recurrence is poor.

Poor outcomes are observed in patients with Denys-Drash syndrome as the disease is often bilateral and associated with progressive renal failure.[63][101]​​[143] However, overall survival is improved with successful renal transplant.[144]

One study found that Hispanic ethnicity may be associated with poorer prognosis; however, further research on ethnic disparities in Wilms tumor is required.[3][145]​​

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